The present invention is in the field of healthcare applications, and relates to a system and method for monitoring disease severity and progression, in particular Amyotrophic Lateral Sclerosis (ALS) disease.
ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. The progressive degeneration of the motor neurons, which reach and activate the muscles throughout the body, eventually leads to their demise. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients gradually lose their ability to speak, eat, move and breathe.
There are two different causes for ALS, sporadic and familial. Sporadic, which is the most common form of the disease, may affect anyone, anywhere. Familial ALS, which is inherited, accounts for 5 to 10 percent of all cases. In familial ALS, there is a 50% chance each offspring will inherit the mutated gene and develop the disease.
The gold standard assay to monitor disease progression is a subjective questionnaire, designated ALSFRS (ALS Functional Rating Scale), The questionnaire is answered by the patient and/or his doctor during routine examination, usually once in 3 months.
While scientists are making progress in exploring the disease causes and molecular mechanism, no cure for ALS is known to date. The average survival from onset to death is three to five years. Only about 10% of the patients survive longer than years. The only FDA-approved medication, Riluzole, may slow disease progression and extend life expectancy by several months in some patients.